Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Subacute sclerosing panencephalitis (SSPE) is a rare central nervous system degenerative disease that occurs primarily in children and adolescents. It is believed to be caused by a measles-like virus. Initial symptoms usually present as a variety of personality changes followed by myoclonus with progression of mental and motor deterioration, which leads to death within a few months to years. New experimental treatment with inosiplex has been shown to be helpful for patients stricken with this progressive neurological disease. A response to inosiplex therapy is best in patients with a slowly progressing form of the disease. Inosiplex treatment is safe with few adverse effects. The duration of treatment appears to be lifelong since many patients relapse when inosiplex therapy is discontinued. This article reviews the etiology, pathogenesis, and experimental treatment of SSPE.
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PMID:Treatment of subacute sclerosing panencephalitis: an overview. 620 74

Myoclonus is a characteristic neurological sign of subacute sclerosing panencephalitis (SSPE). Attempts were made to induce myoclonus in a large proportion of hamsters with a cell-associated strain of SSPE virus (the Biken strain) and thereby to establish an experimental model for study of the mechanism of development of this condition. When injected intracerebrally, Biken virus induced myoclonus within two to 14 days in 84% of the three- to nine-week-old hamsters tested. Electroencephalographic traces showed a periodic and synchronous discharge consisting of high-voltage slow waves and spikes that appeared coincidentally with myoclonus. Neurons in the cortex and thalamus of the affected animals had severely degenerated cytoplasm. Inflammatory changes, such as perivascular cuffing or infiltration of mononuclear cells, were not detected. Staining with immunoperoxidase revealed measles viral antigens in the cytoplasm and dendrites of the affected neurons. SSPE virus with the same properties as the parent virus was recovered from brain cells of sick animals by cocultivation with Vero cells.
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PMID:Induction of acute myoclonic encephalopathy in hamsters by subacute sclerosing panencephalitis virus. 648 Nov 83

Cell-free viruses recovered from virus-carrying cultures of the Niigata-1, Kitaken-1, and Biken strains of SSPE virus were examined for neurovirulence. The cell-free viruses were prepared by freezing and thawing or by EDTA treatment of the virus-carrying cultures and inoculated into adult mice intracerebrally. A considerable number of the inoculated mice showed clinical signs about 1 to 5 weeks after the inoculation. The first symptom was hyperreactivity, which was followed by paresis and myoclonus. All of the affected mice fell in paralysis and finally died. The virus could be recovered from the moribund mice by cocultivation of the brain cells with Vero cells. Immunofluorescence staining of the brain tissue revealed that infected cells containing viral antigens were distributed sparsely. No inflammatory feature, however, was observed in the brain as far as examined and neutralizing antibody against SSPE virus was not detected in sera from the mice inoculated with the cell-free SSPE viruses.
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PMID:Mode of subacute sclerosing panencephalitis (SSPE) virus infection in tissue culture cells. III. Neurovirulence of cell-free SSPE viruses of Niigata-1, Kitaken-1, and Biken strains. 731 88

Thirty-eight cases of subacute sclerosing panencephalitis (SSPE) were reviewed. Deterioration in school performance, personality changes, and seizures were common early symptoms. Initial examination frequently showed myoclonus, spasticity, and extrapyramidal dysfunction, and in two-thirds of patients these findings were asymmetrical or focal. Retinitis or papilledema was present on initial examination in 50% of the patients. At last follow-up 24 children had died, with a mean survival of 42 months. Most patients reached a state of severe neurological impairment within 13 months. Subsequent evidence of improvement was noted in 10 children and was sustained in 4. Fifteen patients received antiviral treatment. Ten treated patients died from 5 to 133 months (mean, 58) from onset of their illness, while 15 untreated patients survived a mean of 33 months. Duration of survival appeared to be affected most by treatment with amantadine. Three patients treated with the drug were alive 97 to 139 months after onset of SSPE, and 5 died with a mean survival of 78 months. Five of 6 individuals treated with rifampin died after a mean survival of 27 months. Prolonged remissions occurred only in patients treated with amantadine. Although the number of treated individuals was small, our data suggest that amantadine may affect the natural course of SSPE.
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PMID:Review of 38 cases of subacute sclerosing panencephalitis: effect of amantadine on the natural course of the disease. 743 85

The characteristic EEG complexes seen in subacute sclerosing panencephalitis and the periodic myoclonus accompanying them are well known. That either of these can occur unilaterally is less well appreciated. However, the combination of unilateral SSPE complexes and contralateral myoclonus is most unusual. Attention is drawn to such a case in this paper.
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PMID:Unilateral subacute sclerosing panencephalitis complexes with contralateral myoclonus. 746 63

Squirrel monkeys with induced canine distemper virus (CDV) encephalitis showed characteristic clinical signs such as seizures or myoclonus, with EEGs showing periodic synchronized discharge (PSD). Histopathologically, there was gliosis and neuronal degeneration diffusely distributed in both the gray and white matters in the subacute phase, the lesions resembling those found in childhood acute viral encephalitis and subacute sclerosing panencephalitis (SSPE). The auditory brain-stem response (ABR) changes were remarkable in the subacute phase, and the recovery of latency was correlated with survival. The visual evoked potential (VEP) abnormalities disappeared in the acute phase and appeared again with delayed latency in the subacute stage. One monkey which did not show clinical signs but had the pathological changes showed the VEP abnormality. These data suggest that the evoked potentials are helpful in judging the prognosis, and that this model is useful for the analysis of the pathogenesis of viral encephalitis.
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PMID:Electroencephalogram and evoked potentials in the primate model of viral encephalitis. 769 64

A 25-year-old man was admitted following deterioration in behavior and onset of blindness. He soon became comatose and died 6 weeks later. Brain biopsy showed nuclear inclusion bodies resembling viral capsids, astrocytosis and perivascular lymphocytic cuffing but no demyelination. The diagnosis of subacute sclerosing panencephalitis was made on finding: measles virus antigens in both serum and cerebrospinal fluid, the identification of measles RNA sequences in brain tissue by the polymerase chain reaction, and intense, oligoclonal, IgG-banding in the CSF. However, the relatively advanced age of the patient, the absence of myoclonus and the nondistinctive EEG profile lacking synchronous bursts of high-voltage slow and sharp waves, are unusual.
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PMID:[An unusual case of subacute sclerosing panencephalitis]. 774 46

A patient with subacute sclerosing panencephalitis (SSPE) was treated with an intraventricular alpha interferon (IFN-alpha) through an Ommaya reservoir. A 17-year-old boy, who had a history of measles exposure at age 1, showed forgetfulness, difficulties in calculation, reading and writing. Two months later he developed generalized convulsions and myoclonic spasms. He was admitted to the National Saigata Hospital in May 20, 1992. On admission, anti-measles antibody titer in the CSF was 1:16 by complement-fixation method. His EEG revealed a periodic synchronous discharge. Therefore, the diagnosis of SSPE was confirmed. An Ommaya reservoir was implanted on July 7, 1992, and an intraventricular administration of INF-alpha was begun after two weeks. The dose of INF-alpha was gradually increased from 1.0 x 10(6) IU/m2 to 2.0 x 10(6) IU/m2 twice a week. Fever, vomiting and anorexia were developed when the INF-alpha injection was first started. When he received a total dose of 8.0 x 10(6) IU, he became bed ridden for remarkable lethargy. The lethargy was continued for about 10 days despite the therapy was interrupted, and then he gradually became alert. The frequency of myoclonus became more frequent and mentality got worse, so the treatment with INF-alpha was tried again in decreasing the dose to 1.0 x 10(6) IU/m2 twice a week. However, be became drowsy again after he received a total of 7.5 x 10(6) IU. With intramuscular or intravenous administrations of the high doses of INF-alpha (> or = 1.0 x 10(7) IU), significant neurological abnormalities were reported to occur.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of subacute sclerosing panencephalitis treated with intraventricular interferon--the side effects of interferon-alpha to the central nervous system]. 815 18

Dyskinesia, here, is considered to be synonymous with abnormal involuntary movement (AIM). AIMs are recognized in various infections of the central nervous system, of which Creutzfeldt-Jakob disease (CJD) and subacute sclerosing panencephalitis (SSPE) are thought to be the most important. The two show myoclonus and other similar features in common except age of onset. The myoclonus is classified under the non-stimulus-sensitive and periodic one characterized by a long duration and a relationship to periodic synchronous discharge (PSD) in EEG. The rhythm is, however, somewhat different and quicker in CJD (0.5-2 Hz) than in SSPE (0.05-0.2 Hz). In the acquired immunodeficiency syndrome (AIDS), AIMs, including tremor, myoclonus and some others are also noted.
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PMID:[Dyskinesias in infections of the central nervous system]. 827 70

Subacute sclerosing panencephalitis (SSPE) had largely disappeared from the United States because of nearly universal measles vaccination, but it has reemerged in children infected with human immunodeficiency virus (HIV). Two children with SSPE are described. The first was HIV positive and presented with seizures and encephalopathy at the age of 21 months. The second developed myoclonus and dementia at age 4 years; she was not infected with HIV, but her mother had acquired immunodeficiency syndrome. Magnetic resonance imaging findings were nonspecific and could have been compatible with HIV encephalopathy. Electroencephalography was characteristic of SSPE, showing high-voltage, periodic slow-wave complexes and background slowing. The diagnosis of SSPE was confirmed by brain biopsy or high measles antibody titers in the cerebrospinal fluid.
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PMID:Subacute sclerosing panencephalitis and acquired immunodeficiency syndrome: role of electroencephalography and magnetic resonance imaging. 863 87


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